Envelope following responses for hearing diagnosis: Robustness and methodological considerations.

Recent studies have found that envelope following responses (EFRs) are a marker of age-related and noise- or ototoxic-induced cochlear synaptopathy (CS) in research animals. Whereas the cochlear injury can be well controlled in animal research studies, humans may have an unknown mixture of sensorineural hearing loss [SNHL; e.g., inner- or outer-hair-cell (OHC) damage or CS] that cannot be teased apart in a standard hearing evaluation. Hence, a direct translation of EFR markers of CS to a differential CS diagnosis in humans might be compromised by the influence of SNHL subtypes and differences in recording modalities between research animals and humans. To quantify the robustness of EFR markers for use in human studies, this study investigates the impact of methodological considerations related to electrode montage, stimulus characteristics, and presentation, as well as analysis method on human-recorded EFR markers. The main focus is on rectangularly modulated pure-tone stimuli to evoke the EFR based on a recent auditory modelling study that showed that the EFR was least affected by OHC damage and most sensitive to CS in this stimulus configuration. The outcomes of this study can help guide future clinical implementations of electroencephalography-based SNHL diagnostic tests.

Development of the hearing-related quality of life questionnaire for auditory-visual, cognitive and psychosocial functioning (hAVICOP).

INTRODUCTION: There is a need for a validated and standardized self-assessment instrument to assess the subjective effect of hearing aid (HA) use and/or cochlear implantation (CI) on different aspects of functioning in daily life. The aim of this study was to develop a new holistic Patient Reported Outcome Measure (PROM) to assess hearing-related quality of life. The new PROM is titled the hearing-related quality of life questionnaire for Auditory-VIsual, COgnitive and Psychosocial functioning (hAVICOP). METHODS: A conceptual framework was set up and test items were prepared per domain. Preliminary testing involved a semi-structured interview-based assessment in normal-hearing and hearing-impaired adults and an expert panel. For the further psychometric evaluation, a new sample of 15 adult HA users, 20 adult CI users and 20 normal-hearing adults filled in the refined version of the hAVICOP, the Speech, Spatial and Qualities of Hearing Scale, the Nijmegen Cochlear Implant Questionnaire and the TNO-AZL Questionnaire for Adult's Health-Related Quality of Life. Based on these results, a factor analysis was conducted and internal consistency, discriminant validity and concurrent construct validity were determined. RESULTS: The final version of the hAVICOP consists of three domains for hearing-related quality of life: (1) auditory-visual functioning, (2) cognitive functioning, and (3) psychosocial functioning. A sufficient internal consistency was found, and discriminant validity and concurrent construct validity were good. CONCLUSIONS: A new PROM to assess hearing-related quality of life was developed, named the hAVICOP. In the future the validity and reliability should be examined further.

Binaural interaction in the auditory brainstem response: a normative study.

OBJECTIVE: Binaural interaction can be investigated using auditory evoked potentials. A binaural interaction component can be derived from the auditory brainstem response (ABR-BIC) and is considered evidence for binaural interaction at the level of the brainstem. Although click ABR-BIC has been investigated thoroughly, data on 500 Hz tone-burst (TB) ABR-BICs are scarce. In this study, characteristics of click and 500 Hz TB ABR-BICs are described. Furthermore, reliability of both click and 500 Hz TB ABR-BIC are investigated. METHODS: Eighteen normal hearing young adults (eight women, ten men) were included. ABRs were recorded in response to clicks and 500 Hz TBs. ABR-BICs were derived by subtracting the binaural response from the sum of the monaural responses measured in opposite ears. RESULTS: Good inter-rater reliability is obtained for both click and 500 Hz TB ABR-BICs. The most reliable peak in click ABR-BIC occurs at a mean latency of 6.06 ms (SD 0.354 ms). Reliable 500 Hz TB ABR-BIC are obtained with a mean latency of 9.47 ms (SD 0.678 ms). Amplitudes are larger for 500 Hz TB ABR-BIC than for clicks. CONCLUSION: The most reliable peak in click ABR-BIC occurs at the downslope of wave V. Five hundred Hertz TB ABR-BIC is characterized by a broad positivity occurring at the level of wave V. SIGNIFICANCE: The ABR-BIC is a useful technique to investigate binaural interaction in certain populations. Examples are bilateral hearing aid users, bilateral cochlear implant users and bimodal listeners. The latter refers to the combination of unilateral cochlear implantation and contralateral residual hearing. The majority of these patients have residual hearing in the low frequencies. The current study suggests that 500 Hz TB ABR-BIC may be a suitable technique to assess binaural interaction in this specific population of cochlear implant users.

Hearing impairment in Stickler syndrome: a systematic review.

BACKGROUND: Stickler syndrome is a connective tissue disorder characterized by ocular, skeletal, orofacial and auditory defects. It is caused by mutations in different collagen genes, namely COL2A1, COL11A1 and COL11A2 (autosomal dominant inheritance), and COL9A1 and COL9A2 (autosomal recessive inheritance). The auditory phenotype in Stickler syndrome is inconsistently reported. Therefore we performed a systematic review of the literature to give an up-to-date overview of hearing loss in Stickler syndrome, and correlated it with the genotype. METHODS: English-language literature was reviewed through searches of PubMed and Web of Science, in order to find relevant articles describing auditory features in Stickler patients, along with genotype. Prevalences of hearing loss are calculated and correlated with the different affected genes and type of mutation. RESULTS: 313 patients (102 families) individually described in 46 articles were included. Hearing loss was found in 62.9%, mostly mild to moderate when reported. Hearing impairment was predominantly sensorineural (67.8%). Conductive (14.1%) and mixed (18.1%) hearing loss was primarily found in young patients or patients with a palatal defect. Overall, mutations in COL11A1 (82.5%) and COL11A2 (94.1%) seem to be more frequently associated with hearing impairment than mutations in COL2A1 (52.2%). CONCLUSIONS: Hearing impairment in patients with Stickler syndrome is common. Sensorineural hearing loss predominates, but also conductive hearing loss, especially in children and patients with a palatal defect, may occur. The distinct disease-causing collagen genes are associated with a different prevalence of hearing impairment, but still large phenotypic variation exists. Regular auditory follow-up is strongly advised, particularly because many Stickler patients are visually impaired.

Clinical evaluation of cochlear implant sound coding taking into account conjectural masking functions, MP3000™.

Efficacy of the SPEAK and ACE coding strategies was compared with that of a new strategy, MP3000™, by 37 European implant centers including 221 subjects. The SPEAK and ACE strategies are based on selection of 8-10 spectral components with the highest levels, while MP3000 is based on the selection of only 4-6 components, with the highest levels relative to an estimate of the spread of masking. The pulse rate per component was fixed. No significant difference was found for the speech scores and for coding preference between the SPEAK/ACE and MP3000 strategies. Battery life was 24% longer for the MP3000 strategy. With MP3000 the best results were found for a selection of six components. In addition, the best results were found for a masking function with a low-frequency slope of 50 dB/Bark and a high-frequency slope of 37 dB/Bark (50/37) as compared to the other combinations examined of 40/30 and 20/15 dB/Bark. The best results found for the steepest slopes do not seem to agree with current estimates of the spread of masking in electrical stimulation. Future research might reveal if performance with respect to SPEAK/ACE can be enhanced by increasing the number of channels in MP3000 beyond 4-6 and it should shed more light on the optimum steepness of the slopes of the masking functions applied in MP3000.

Determination and evaluation of clinically efficient stopping criteria for the multiple auditory steady-state response technique.

OBJECTIVE: The present study aimed at establishing clinically efficient stopping criteria for a multiple 80-Hz auditory steady-state response (ASSR) system. METHODS: In Experiment 1, data of 31 normal-hearing subjects were analyzed off-line to propose stopping rules. Consequently, ASSR recordings will be stopped when (1) all 8 responses reach significance and significance can be maintained for 8 consecutive sweeps; or (2) the mean noise levels were 4nV (if p-values were between 0.05 and 0.1, measurements were extended once by 8 sweeps); or (3) a maximum amount of 48 sweeps was attained; whichever occurred first. In Experiment 2, these stopping criteria were applied on 10 normal hearing and 10 hearing-impaired adults to assess the efficiency. RESULTS: The application of these stopping rules resulted in ASSR threshold values that were comparable to other ASSR research. Furthermore, preliminary analysis of the response and noise amplitudes demonstrated slightly higher values for hearing impaired than normal-hearing subjects. CONCLUSIONS: The proposed stopping rules can be used in adults to determine accurate ASSR thresholds within a time-frame of about 1h. SIGNIFICANCE: The use of these a priori stopping criteria might assist the clinician in their decision to terminate ASSR recordings.

Forward-masking patterns produced by symmetric and asymmetric pulse shapes in electric hearing.

Two forward-masking experiments were conducted with six cochlear implant listeners to test whether asymmetric pulse shapes would improve the place-specificity of stimulation compared to symmetric ones. The maskers were either cathodic-first symmetric biphasic, pseudomonophasic (i.e., with a second anodic phase longer and lower in amplitude than the first phase), or "delayed pseudomonophasic" (identical to pseudomonophasic but with an inter-phase gap) stimuli. In experiment 1, forward-masking patterns for monopolar maskers were obtained by keeping each masker fixed on a middle electrode of the array and measuring the masked thresholds of a monopolar signal presented on several other electrodes. The results were very variable, and no difference between pulse shapes was found. In experiment 2, six maskers were used in a wide bipolar (bipolar+9) configuration: the same three pulse shapes as in experiment 1, either cathodic-first relative to the most apical or relative to the most basal electrode of the bipolar channel. The pseudomonophasic masker showed a stronger excitation proximal to the electrode of the bipolar pair for which the short, high-amplitude phase was anodic. However, no difference was obtained with the symmetric and, more surprisingly, with the delayed pseudomonophasic maskers. Implications for cochlear implant design are discussed.

The effects of aging on evoked otoacoustic emissions and efferent suppression of transient evoked otoacoustic emissions.

OBJECTIVE: There is still controversy regarding the effects of aging on evoked otoacoustic emissions (EOAEs), as well as on the efferent system measured by contralateral acoustic stimulation of EOAEs. The purpose of this study was to investigate the deterioration in EOAEs and efferent suppression (ES) in a representative sample statistically controlling for the differences in hearing thresholds. METHODS: Seventy-one ears (20-79years) were included in the study, 47 of which had normal hearing thresholds, and 24 ears had a sensorineural high-frequency hearing loss caused by presbycusis. The effects of aging on transient evoked (TEOAEs) and distortion product OAEs (DPOAEs), and on ES were evaluated using multiple regression and correlation coefficients. RESULTS: EOAEs and ES were more strongly correlated with age, than with pure-tone thresholds (PTTs). Moreover, the increase in the amount of variance explained by the regression model using both predictors was larger for PTTs as compared to the variable age. CONCLUSIONS: The deterioration of EOAEs and ES with advancing age is caused mainly by pure age-effects, and additionally by the reduction in hearing thresholds. SIGNIFICANCE: The relative contribution of age and hearing thresholds on EOAEs, as well as on ES is important for their interpretation in clinical settings.

The clinical value of the multiple-frequency 80-Hz auditory steady-state response in adults with normal hearing and hearing loss.

OBJECTIVES: To determine the ability of the air-conduction multiple-frequency auditory steady-state response (ASSR) technique to diagnose normal hearing (NH) and mild and moderate degrees of sensorineural hearing loss (SNHL), to assess patients with conductive hearing loss (CHL), to evaluate flat and sloping configurations of hearing impairment, and to provide sensitivity and specificity values for various ASSR cutoff criteria. DESIGN: A comparative study between ASSR and criterion-standard behavioral thresholds. SETTING: Ear, nose, and throat department at a university hospital. PATIENTS: The study population comprised 40 adults with NH, 17 with SNHL, and 7 with CHL. MAIN OUTCOME MEASURES: The measure of interest was the difference between ASSR and behavioral thresholds at 0.5, 1.0, 2.0, and 4.0 kHz. The sensitivity, specificity, positive predictive value, negative predictive value, and efficiency were calculated for several ASSR cutoff criteria. RESULTS: The ASSR technique clearly distinguished moderate SNHL from NH, but the "mild SNHL and NH" and "mild SNHL and moderate SNHL" differentiation was particularly difficult at 0.5 and 2.0 kHz, respectively. Air-conduction ASSR thresholds accurately predicted behavioral thresholds in CHL. The ASSR system precisely reflected the flat and sloping configurations. Finally, the most appropriate ASSR cutoff point for normality seems to be the 30-dB-or-lower criterion. CONCLUSIONS: In adults, the multiple-frequency 80-Hz ASSR technique can be used to determine the degree and configuration of hearing loss. Although air-conduction ASSR thresholds accurately predicted behavioral thresholds in CHL, future research with bone-conduction ASSRs is necessary to establish the type of hearing loss. Furthermore, the applicability of these findings still needs to be confirmed for infants.

Auditory steady-state responses to MM and exponential envelope AM(2)/FM stimuli in normal-hearing adults.

The present study utilized a commercially available multiple auditory steady-state response (ASSR) system to test normal hearing adults (n=55). The primary objective was to evaluate the impact of the mixed modulation (MM) and the novel proposed exponential AM(2)/FM stimuli on the signal-to-noise ratio (SNR) and threshold estimation accuracy, through a within-subject comparison. The second aim was to establish a normative database for both stimulus types. The results demonstrated that the AM(2)/FM and MM stimulus had a similar effect on the SNR, whereas the ASSR threshold results revealed that the AM(2)/FM produced better thresholds than the MM stimulus for the 500, 1000, and 4000 Hz carrier frequency. The mean difference scores to tones of 500, 1000, 2000, and 4000 Hz were for the MM stimulus: 20+/-12, 14+/-9, 10+/-8, and 12+/-8 dB; and for the AM(2)/FM stimulus: 18+/-13, 12+/-8, 11+/-8, and 10+/-8 dB, respectively. The current research confirms that the AM(2)/FM stimulus can be used efficiently to test normal hearing adults.

Pneumococcal conjugate vaccination in children with recurrent acute otitis media: a therapeutic alternative?

BACKGROUND: Based on two clinical trials in healthy infants the American Academy of Pediatrics (AAP) advices immunization with a 7-valent pneumococcal conjugate vaccine in children with recurrent acute otitis media (AOM). OBJECTIVE: To study the efficacy of a 7-valent pneumococcal conjugate vaccine on acute otitis media recurrences, its immunogenicity and impact on nasopharyngeal Streptococcus pneumoniae carriage in children with a history of frequent acute otitis media. METHODS: In this double-blind, randomized study, 74 Belgian children, aged 1-7 years, with at least 2 clinically diagnosed episodes of acute otitis media in the previous year were enrolled. Children were immunized with either a 7-valent pneumococcal conjugate vaccine followed by a 23-valent pneumococcal polysaccharide booster or a control hepatitis A vaccine. Total follow-up was 26 months. RESULTS: Despite adequate serum IgG responses to all conjugate vaccine pneumococcal serotypes, no reduction of acute otitis media episodes was observed in the pneumococcal vaccine group as compared to the control group (rate ratio: 1.16; 95% CI: 0.69-1.96). Overall nasopharyngeal pneumococcal carriage remained stable. However, a transient shift from conjugate vaccine related S. pneumoniae serogroups to non-vaccine related serogroups was noted following conjugate vaccination. CONCLUSION: Clinically no protective effect of pneumococcal conjugate vaccination on acute otitis media recurrences was found in children with a history of frequent AOM.